European Journal of Pediatrics

, Volume 170, Issue 4, pp 445–452 | Cite as

The Eurofever Project: towards better care for autoinflammatory diseases

  • Seza OzenEmail author
  • Joost Frenkel
  • Nicola Ruperto
  • Marco Gattorno
  • on behalf of the Eurofever Project


Autoinflammatory diseases are a group of diseases characterized by inflammatory attacks. The monogenic forms of these diseases are also classified as the hereditary periodic fever syndromes. All are characterized by attacks of fever along with certain clinical features and high acute phase reactants. Most of these monogenic diseases are associated with hereditary disorders of the interleukin-1 pathway. The most common autoinflammatory disease is familial Mediterranean fever. The other rather common monogenic diseases are the tumor necrosis factor receptor-associated periodic syndrome, hyperimmunoglobulinemia D with periodic fever syndrome, and cryopyrin-associated periodic fever syndromes (CAPS). However, a number of multifactorial diseases such as Behçet disease are now also categorized under the topic of autoinflammatory diseases. The main features and management of these diseases will be reviewed. Finally, we introduce the “Eurofever” project, aimed to increase awareness and education for the aforementioned diseases. We conclude that the pediatrician should be aware of the features and management of autoinflammatory diseases since all present with fever—the most common symptom of pediatric practice.


Autoinflammatory diseases Familial Mediterranean fever Tumor necrosis factor receptor-associated periodic syndrome Hyperimmunoglobulinemia D with periodic fever syndrome Cryopyrin-associated periodic fever syndromes 



This paper was supported by the Eurofever Project by the Executive Agency for Health and Consumers (EAHC, Project No. 2007332) and EUROTRAPS (FP7 project n. HEALTH-F2-2008-200923).


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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Seza Ozen
    • 1
    Email author
  • Joost Frenkel
    • 2
  • Nicola Ruperto
    • 3
  • Marco Gattorno
    • 3
  • on behalf of the Eurofever Project
  1. 1.Department of Pediatrics, Faculty of MedicineHacettepe UniversityAnkaraTurkey
  2. 2.Department of PediatricsUniversity of UtrechtUtrechtThe Netherlands
  3. 3.UO Pediatria II—Reumatologia“G. Gaslini” InstituteGenoaItaly

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